[Psuedo primary hypokinetic cardiopathies and diffuse coronary atherosclerosis. Silent ischemic cardiomyopathies].
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Biomedical subjects
Publications and source records attributed to J L Neimann.
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This joint work has studied the cardiomyopathies occurring in hereditary neuro-muscular disorders (270 cases). The Duchenne type of disorder (74 cases) was responsible for asystole (4 cases), for cardiomegaly, and especially for abnormalities of the ECG (59 cases)--Q waves and large R waves in V1 and V6. The cardiomyopathy was of the hypokinetic type, with histological evidence of degeneration of the myocardial fibres. Dystrophia myotonica of Steinart (23 cases) caused conductive disorders (17 cases) which were either atrioventricular or intra-ventricular or both. Studies of the His pathway confirmed that these abnormalities were more diffuse in 5 cases. The main histological feature was interstitial fibrosis. There was a high risk of sudden death; ECG follow-up should be close. Friedreich's disease (20 cases) in its complete form led to later development of obstructive cardiomyopathy, with a systolic ejection murmur, cardiomegaly, and abnormalities of the ECG--left ventricular hypertrophy in the vertical axis, right ventricular and septal hypertrophy, repolarisation disorders similar to those found in coronary artery disease. Histology showed hypertrophy with degeneration of the myocardial fibres and interstitial fibrosis. This complete form was rare (7 cases out of 20); on the other hand, ECG abnormalites were very common (16 cases out of 20). The authors have tried to study the relationships between primary cardiomyopathies (50 cases) and peripheral neuromuscular disorders. 17 of the 39 peripheral muscle biopsies were abnormal, but a well-defined muscular dystrophy could not be found in them.
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Using monoplanar angiography at 30 degrees, the authors have attempted to quantify the effectiveness of the non-aneurysmal myocardium in 50 patients (17 of them having undergone surgery) with an aneurysm of the left ventricle. Having fixed the position of two points during systole and diastole, they first delimit the aneurysmal zone and/or neighbouring dyskinetic zones in relation to the zones which are still 'healthy'. By a method of integration based on the formula of the three levels, they first measure the global volume of the left ventricle and its ejection fraction, and gain an idea of the size of the aneurysm. They then evaluate the ejection fraction and the volumes of each of the other zones. They have shown that the prognosis depends upon the ejection fraction and the end-diastolic volume of the ventricle which is presumed to be healthy, these measurements being corrected according to the size, expressed as an ejection volume, of the adjacent dyskinetic zone, which can be assessed by an index (volume of the dyskinetic zone as a proportion of the total 'healthy' and dyskinetic zones, in turn expressed as a ratio of the ejection fraction). The application of this work is to be found in seeking a limit of operability for extensive left ventricular aneurysms with cardiac failure.
The authors compare 5 methods of evaluating left ventricular volume and the ejection fraction by monoplanar angiocardiography in the 30 degrees right oblique incidence: the method of Green (1 and 2), Snow and Dodge, and by trapezoidal integration which was used as a reference. The have calculated the regression ordinates and the correlation coefficients for various systolic and diastolic volumes as well as for various ejection fractions. For these latter, Dodge's quadratic equation can also be used. Provided there is no gross distorsion of ventricular contolr, there is fairly good correlation, but there is a marked discrepancy for large volumes or small ejection fractions (less than 0.40). When more elaborate methods are not available, it seems reasonable to use a single method to calculate these parameters, but it is essential to have recourse to other techniques when there are gross changes in the left ventricle.
The authors have studied the exercise test carried out at least three months (3-6 months: 52 cases; greater than 6 months: 48 cases) after myocardial infarction in the anterior position (50 cases), in the "inferior" position (42 cases), and of a diffuse type (8 cases), in patients who were taking no treatment which might interfere with interpretation of the test. For the anterior infarctions there was a good correlation between ST elevation (J max greater than or equal to 1 mm, or better than the sun of the J greater than or equal to 2 mm) and the presence of severe involvement of the left ventricle. A depressed ST segment beyond the area of necrosis corresponds to a stenosis greater than or equal to 75 p. 100 in 36 p. 100 of cases. In inferior infarctions, the correlation between ST elevation and left ventricular involvement is also specific but less sensitive. ST depression outside the area of necrosis corresponds to a stenosis greater than or equal to 75 p. 100 in 66 p. 100 of cases; it is then lateral, but may extend as far as V2. They also indicate a mirror image, especially when the depressed area slopes upwards, and is localised in V2-V3. The other changes which were found have no practical application.
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The various types of anomalous origin and unusual course and/or predominance of the coronary arteries encountered in a review of 3 000 selective coronary arteriograms were studied. 1. Abnormal ostia: Ectopic ostia with anterior, posterior or lateral displacement (11 cases). Variations in the number of ostia:--accessory ostium for the conus branch (68 cases);--distinct ostia for LAD and circumflex artery (21 cases);--single coronary artery (6 cases). 2. Abnormal course of coronary arteries: Anomalous circumflex origin from the right coronary artery (8 cases). Unusual predominance of the left coronary artery:--right predominance 74,5 %;--left predominance 14 %;--balanced 11,5 %. In aortic stenosis, the frequency of these different malformations is very different. Different patterns of vascularizattion of the centers of automatism and of the left ventricular free wall were also studied.
A selective coronary arteriography was performed in 30 subjects with myocardial infarction, after disappearance of the necrosis wave. The angiographic characteristics of this group when compared with a series of infarctions, unselected by the clinical course, wers: a decreased severity index; a better development of the compensatory circulation; and a lesser frequency of severe disturbances of ventricular dynamics. Disappearance of the Q wave might be explained by a very distal occlusion in 2 cases, by a recanalization in 4 cases and by a very intense development of the collateral circulation in 8 cases. One subject had a normal coronary arteriography.
Based on the study of 25 cases, the authors define menace syndrome in the anterior descending artery (ADA) according to three criteria: angina of recent onset and rapid development, a major disorder of repolarisation in the anterior part of the heart, and a tight proximal stenosis of the ADA. Of seven patients who could not undergo surgery, three sustained anterior infarctions, and three died after 30 days. 18 patients underwent surgery to revascularise the myocardium by anastomosis of the internal mammary artery to the ADA; 13 of these were carried out as urgent procedures (an average of 26 hours after coronary arteriography). The functional results were excellent, and all of the internal mammary anastomoses studied at 6 months (9 cases) were patent. This study has shown that menace syndrome of the ADA is a surgical emergency, and that a procedure to anastomose the coronary arteries should be carried out immediately after coronary anteriography.
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This study, based on 39 cases, confirms the value of two-dimensional echocardiography in the diagnosis of right ventricular infarction during the acute phase. This diagnosis is confirmed by the presence of abnormalities of the segmental kinetics always present in the inferior wall and less frequently in the anterior wall. The inferior part of the septum is affected in two thirds of cases. Right ventricular dilation is frequently observed, but is not constant. Two-dimensional echocardiography has a diagnostic sensitivity similar to that of isotope ventriculography, but greater than that of haemodynamic investigations. Finally, apart from the easy demonstration of complications (tricuspid incompetence, septal rupture, intracardiac thrombosis), two dimensional echocardiography can be used to evaluate right ventricular function.
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